- Email: [email protected]
Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy
Shu-Chien Huang, MD, PhD, En-Ting Wu, MD, PhD, Shyh-Jye Chen, MD, Chi-Hsiang Huang, MD, Jin-Chung Shih, MD, PhD, Hen-Wen Chou, MD, Chung-I Chang, MD, Ing-Sh Chiu, MD, PhD, and Yih-Sharng Chen, MD, PhD Departments of Surgery, Pediatrics, Medical Imaging, Anesthesiology, and Obstetrics and Gynecology, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan
Background. Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. Methods. Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n [ 5), and a systemic-pulmonary shunt was added if needed (n [ 3). Results. All patients presented with intractable heart failure or severe cyanosis requiring mechanical
ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n [ 2) and tetralogy of Fallot (n [ 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years). Conclusions. Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.
M
Conversely, biventricular repair was proposed for symptomatic neonates with Ebstein anomaly by KnottCraig and colleagues [10–12] and some limited case reports from other centers [13–15]. The technique of tricuspid valvuloplasty (TVP) for Ebstein anomaly evolved over time [16, 17]. The introduction of cone reconstruction [18, 19] significantly improved surgical results and has become a widely accepted technique for children and adults [12, 14, 20], but TVP has not been carried out much in neonates. The difficulty of neonatal Ebstein anomaly repair lies not only in the delicate surgical techniques for repairing a neonatal TV, but also in overcoming the high pulmonary vascular resistance (PVR) in neonatal physiology. Since 2007 we have performed TV repair for neonatal Ebstein anomaly. Here we describe our surgical strategy aiming for biventricular repair.
anagement of a neonate with symptomatic Ebstein anomaly is a very challenging situation, and it carries a high risk of early death [1–6]. Tricuspid valve (TV) repair for symptomatic neonatal Ebstein anomaly is reportedly an extremely high-risk procedure, especially in patients with Ebstein anomaly and right ventricular (RV) outflow obstruction [4, 7, 8]. In 1991 the Starnes operation was introduced for severe neonatal Ebstein anomaly. This procedure involved the univentricularization of the heart by TV closure using a fenestrated patch and a modified Blalock-Taussig shunt (BT shunt) [9]. The Starnes operation as a method of single ventricle palliation is advocated for relatively simple operations with acceptable survival rates for neonates or young infants with Ebstein anomaly. Accepted for publication Jan 18, 2017. Presented at the Sixty-third Annual Meeting of the Southern Thoracic Surgical Association, Naples, FL, Nov 9–12, 2016. Address correspondence to Dr Huang, Department of Surgery, National Taiwan University Hospital, 7 Chung-Shan South Rd, Taipei 100, Taiwan; email: [email protected].
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier Inc.
(Ann Thorac Surg 2017;104:917–25) Ó 2017 by The Society of Thoracic Surgeons
Patients and Methods Between 2007 and 2016, 7 severely symptomatic neonates (n ¼ 5) or young infants (n ¼ 2) with Ebstein anomaly 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2017.01.081
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Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy
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underwent TV repair (TVP) operations at the National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. We did not have experience with the Starnes operation in this cohort. The patients with pulmonary atresia (PA), intact ventricular septum, severe hypoplastic right ventricle, and small tricuspid annulus (Z score <-2) with Ebstein anomaly were managed by single ventricle reconstruction according to the TV Z score. The patients’ medical records and operative notes were reviewed. This study was approved by the Institutional Review Board of National Taiwan University Hospital, and informed consent was waived.
Surgical Technique The operation included TVP, right atrial (RA) reduction, management of RV outflow, and atrial septal defect (ASD) closure or fenestration. The surgical procedure was performed through a standard median sternotomy and cardiopulmonary bypass. The patent ductus arteriosus was ligated after institution of cardiopulmonary bypass. TV repair was performed with the aorta cross-clamped. The basic principle follows the cone reconstruction technique [18]. The technique is aimed at complete surgical delamination and recruitment of all undelaminated leaflet tissue. The anterior leaflet was incised along the annulus and carefully detached from its adherence to the RV free wall, and the most important papillary muscle and its chordae were preserved. The posterior leaflet and the septal leaflets were also detached from their abnormal insertion sites. Then the edge of the TV apparatus was reattached to the true atrial-ventricular TV annulus. The annulus size was determined according to body weight. Saline testing was performed, and sometimes additional annular plication stitches were applied (Fig 1). The associated ventricular septal defect (VSD) was repaired with a Dacron patch (Bard Sauvage Filamentous Knitted Polyester Fabric, Tempe, AZ) in the usual manner. For the particular patient with tetralogy of Fallot, a patch was placed on the RV outflow tract (RVOT) to Fig 1. (A) The tricuspid valve apparatus was detached from the annulus and from its adherence to the right ventricular wall. (B) The tricuspid valve was reattached to the true annulus, thus making a competent valve at its normal position (patient 4).
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relieve the infundibular stenosis after TV repair and VSD repair [21]. This atrialized RV was not plicated. The ASD was closed in 2 patients with Ebstein anomaly and VSD without pulmonary stenosis (PS), and fenestration was kept for the other patients with PS. One piece of RA wall (approximately 2 cm wide) was excised before closure, so the size of the dilated right atrium was reduced. For the 5 patients with PS, the pulmonary valve was preserved in 3 of them because the valve diameter was approximately 5 mm by intraoperative measurement. The other 2 patients with diminutive pulmonary valves received transannular patches. After trials to wean the patients from cardiopulmonary bypass, 3 patients received additional BT shunts for cyanosis. We routinely delayed sternum closure until the patients were in stable condition. Inotropic agents, including low-dose dopamine, milrinone, and sometimes epinephrine, were used for postoperative RV failure, and inhalation nitric oxide was administered to decrease PVR.
Data Analysis Descriptive statistics were used. Data are expressed as the mean standard deviation or the median and range as appropriate. The end point of the study was early death, defined as death before hospital discharge or within 30 days of the surgical procedure.
Results The patients’ cases are summarized in Table 1.The mean weight of the patients at the time of operation was 2.9 0.7 kg, ranging from 2.0 to 3.7 kg, and the mean age at the time of surgical intervention was 31 30 days. The diagnosis had been made prenatally in 5 (71%) of the patients. Two patients had associated VSD, and the indication for operation was intractable heart failure. One patient had associated tetralogy of Fallot and presented with severe heart failure and severe cyanosis. The other 4 patients had PS or functional PA and presented with severe cyanosis, with 1 patient requiring immediate
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Table 1. Demographic Features, Echocardiographic Findings, and Clinical Presentation of the 7 Patients With Ebstein Anomaly CXR
Echocardiographic Findings
TV Downward Patient Age Displacement No. (days) BW (kg) C/T Ratio (mm) 23
3.1
0.76
7.3
2
102
3.4
0.7
12.8
3
18
2.7
0.84
6.9
4
36
3.4
0.88
16.3
5
6
2
0.91
6
11
2.1
0.83
7
27
3.7
0.79
Celermajer Index [2]
RVOT Flow
Pulmonary Annulus Carpentier (mm) Classification
Associated Lesions
Preoperative Condition
Moderate (51 mm Hg) Severe (58 mm Hg)
0.7
No PS
10
C
VSD, perimembranous NIPPV, heart failure
1.01
No PS
8
C
VSD, muscular trabecular, aberrant RSCA
Severe (34 mm Hg) Severe (8 mm Hg)
0.98
Poor antegrade flow, moderate PR No antegrade flow
10
B–C
Valvular PS
7.1
C–D
9.1
Severe (34 mm Hg)
1.5
No antegrade flow, PR(þ)
6.5
C
Functional pulmonary atresia Severe PS
5.7
Severe (90 mm Hg) Severe (14 mm Hg)
1.34
Scant antegrade flow
6.3
B
Critical PS
1.27
PS, 10 mm Hg
5.3
C
Tetralogy of Fallot, Ventilator, cyanosis, heart mild RV hypoplasia failure
15
1.85
Status after PDA ligation, prematurity, acute renal failure, subglottic stenosis, Ventilator, heart failure Ventilator, cyanosis Cyanosis after shunt, ventilator, heart failure Prematurity, hydrops fetalis, ECMO immediately after birth Ventilator, iNO, shock
BW ¼ body weight; C/T ratio ¼ cardiothoracic ratio; CXR ¼ chest roentgenogram; ECMO ¼ extracorporeal membrane oxygenation; iNO ¼ inhalation nitric oxide; positive pressure ventilation; PDA ¼ patent ductus arteriosus; PR ¼ pulmonary regurgitation; PS ¼ pulmonary stenosis; RSCA ¼ right subclavian artery; RVOT ¼ right ventricular outflow tract; TR ¼ tricuspid regurgitation; TV ¼ tricuspid valve; VSD ¼ ventricular septal defect.
NIPPV ¼ noninvasive RV ¼ right ventricular;
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TR Severity (Pressure Gradient)
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extracorporeal membrane oxygenation (ECMO) support after birth. Two patients underwent surgical procedures before TVP; 1 patient had ligation of the patent ductus arteriosus for heart failure, whereas the other patient received a BT shunt for functional PA and severe cyanosis. Neither patient could be separated from ventilator support before the TVP operation. The operative details and outcomes are summarized in Table 2.
Mortality One death occurred (patient 6). This 2.1-kg girl had a prenatal diagnosis, and she received high-frequency oscillatory ventilation, inhalation nitric oxide, dopamine, and dobutamine for desaturation and shock. She was referred to our hospital, and an emergency operation was performed. Ebstein anomaly with a critical anatomic valvular stenosis (no antegrade flow noted) was found. She received TVP, ASD fenestration, RA reduction, an RVOT patch, and a 3-mm shunt. On the second postoperative day, ECMO was begun for worsening oxygen saturation. The patient was able to be weaned from ECMO with her oxygen saturation at approximately 80% to 90% after we changed the BT shunt from a 3-mm graft to a 3.5-mm graft. However, 2 months later, an episode of severe desaturation and shock required another course of ECMO. Severe tricuspid regurgitation (TR) was noted, and the posterior leaflet was found to be detached as a result of torn stitches. Despite repeat TVP, shunt division, and RVOT reconstruction, she did not recover from renal failure and multiorgan dysfunction and died 1 month after the second operation.
Ventilator Use The median time to extubation was 19 days. Generally, the saturation level gradually improved as the PVR decreased or RV function improved after the surgical procedure in the first postoperative week. One patient (patient 2) struggled for 3 months before accepting operation for Ebstein anomaly with severe TR and VSD. She had subglottic stenosis caused by repeated preoperative intubation and required postoperative tracheostomy. All the other patients were successfully extubated. No patients required supplement oxygen after discharge.
Extracorporeal Membrane Oxygenation One patient (patient 5) received ECMO preoperatively. She had a prenatal diagnosis of Ebstein anomaly with severe TR, pulmonary regurgitation, and hydrops fetalis. She was born by cesarean delivery and had profound edema, cyanosis, and bradycardia. We performed ECMO and patent ductus arteriosus ligation immediately. There was virtually no antegrade pulmonary blood flow, and the main and bilateral pulmonary arteries were less than 2 mm in diameter. After her edematous status improved, we performed TVP, pulmonary valve commissurotomy with a small RVOT patch, and a right BT shunt (3 mm) when she was 6 days old. ECMO was maintained after the
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operation for 5 days. Because of the persistent small right pulmonary artery with juxtashunt stenosis, balloon dilatation of the right pulmonary artery was performed. After a prolonged stay in the intensive care unit, her saturation gradually improved, and she was eventually extubated 92 days postoperatively.
Follow-Up and Reintervention Six patients were discharged with a median follow-up duration of 4.5 years (range, 0.8 to 9.9 years). All 6 patients are currently surviving. The TR was mild in 5 patients and moderate in 1 patient. For the patients with PS (n ¼ 4), 1 had residual moderate PS, and the other 3 had PS well relieved with mild pulmonary regurgitation.
Fate of Fenestrated Atrial Septal Defect The first 2 patients (patients 1 and 2) with VSD had a successful repair procedure without any right-to-left shunt. Patient 4 already had his shunt occluded and a tiny ASD. Patient 6, with tetralogy of Fallot, had a left-toright shunt through an ASD. The 4 patients had complete biventricular circulation, and only regular follow-up for long-term TV function was needed. Patient3 had residual PS and moderate TR and will possibly require reintervention on the pulmonary valve and TV if the condition worsens. Patient 5 had residual right pulmonary artery stenosis after balloon dilatation. We plan to carry out surgical pulmonary arterioplasty and closure of the shunt in the future. These 2 patients (patients 3 and 5) had right-to-left shunts through the fenestrated ASD. In summary, for the 6 survivors, 4 patients had separate biventricular circulations achieved, and 2 remained in mixed circulation (with right-to-left shunt through the ASD). We anticipate that the 2 aforesaid patients can achieve complete biventricular circulation by closure of the ASD. The atrialized right ventricle did not dilate during the follow-up period, and it became part of the functioning right ventricle after TVP in all the survivors. However, minimal paradoxic septal contraction was still noted on this part of the interventricular septum (Fig 2).
Comment In the cohort, we performed TVP in 7 neonates with severe Ebstein anomaly; 6 (85%) patients survived and achieved biventricular circulation. The result was similar to that of the largest series reported, with an early survival rate of 78.1% (25 of 32) for biventricular repair in neonates and infants with Ebstein anomaly [1].
Indications for Early Surgical Intervention: Associated Ventricular Septal Defect or Pulmonary Stenosis or Atresia Not all babies born with Ebstein anomaly require surgical intervention in early infancy. The Toronto group [7] reported that 75% of these patients did not require
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Table 2. Operative Details and Outcomes
1 2 3
4 5
6
TVP, fenestrated ASD, Functional PA, shunt 3.5 mm mild PS: 5 mm TVP, RVOT patch, True PS, 3 mm fenestrated ASD, BT shunt 3 mm Critical valvular PS TVP, RVOT patch, fenestrated ASD, shunt 3 mm TVP, tetralogy of Fallot Tricuspid, mild repair, fenestrated PS, 5 mm ASD
Follow-Up (y)
146 (101)
7
Survival
9.9
170 (93)
28
Survival
6.5
175 (118)
10
Survival
4.8
106 (69)
24
Survival
4.3
193 (50)
92
Survival
2.5
101 (53)
X
.
152 (124)
14
Postoperative ECMO, change to 3.5 mm shunt, Survival
0.8
TR (PG)
Pulmonary Valve
Mild TR Normal 22 mm Hg Mild TR Normal (24 mm Hg) Mild TR Moderate PS, (34 mm Hg) PG: 36 mm Hg, mild PR Moderate TR Mild PR (32 mm Hg) Mild TR Mild PR (26 mm Hg) .
Mild TR No PS (18 mm Hg)
ASD ¼ atrial septal defect; AXC ¼ aorta cross-clamp time; BT ¼ Blalock-Taussig; CPB ¼ total duration of cardiopulmonary bypass; L / R ¼ left-to-right; PA ¼ pulmonary atresia; PG ¼ pressure gradient; PR ¼ pulmonary regurgitation; PS ¼ pulmonary stenosis; outflow tract; TR ¼ tricuspid regurgitation; TVP ¼ tricuspid valvuloplasty; VSD ¼ ventricular septal defect; X ¼ indicate mortality.
.
ASD
Others
Closed
.
Closed
Tracheostomy for subglottic stenosis .
R/L
Tiny R/L shunt .
Shunt closed at 11 months old Right PA stenosis, shunt partially occluded Death 3 months postoperatively (TR)
L / R shunt
ECMO ¼ extracorporeal membrane oxygenation; R / L ¼ right-to-left; RVOT ¼ right ventricular
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TVP, VSD repair, Normal ASD closure TVP, VSD repair, Normal ASD closure TVP, fenestrated ASD PS(þ) valvular dysplasia
Outcome
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Ventilator Pulmonary Valve CPB (AXC) (Postoperative Patient Operative Procedure (Operative Finding) (min) Days)
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Fig 2. (A) Preoperative and (B) postoperative echocardiograms. The downwardly displaced tricuspid valve was restored to its normal position with a normal appearance. The atrialized right ventricle is shown (arrows) (patient 4).
operations at 1 month old, and similar results were shown in our previous reports [22]. In the presence of a significant VSD, the right ventricle continues to operate at high pressure, thereby magnifying the degree of TR and additional volume overload. Moreover, the presence of a left ventricle (through VSD and TR)–to-RA shunt could further compromise systemic cardiac output and increase systemic venous pressure, thus resulting in intractable heart failure. The presence of PS or PA added a pressure load in addition to the decreased volume of functioning RV. The small functioning RV therefore could not generate enough blood flow to the pulmonary circulation, even if the PVR decreased. In this series, all 7 patients had VSD, PS, or PA. This finding is similar to those of other reported series of neonatal Ebstein anomaly [10, 13]. In the same cohort, other patients with Ebstein anomaly without VSD, PS, or PA did not receive operations in their infancy, and all survived at our institution.
Choice of Operation Severely symptomatic neonates with Ebstein anomaly have an early mortality rate of nearly 100% without surgical intervention [2, 6, 23]. It is still unclear which surgical strategy is the best option for these critically ill neonates. A shunt-only option for patients with Ebstein anomaly with cyanosis or PA has been proposed [5]. However, we had poor results (67% mortality rate) in neonatal Ebstein anomaly in the era before 2007 [22], so we changed this policy. Starnes and colleagues [9, 24, 25] showed that the outcome of TV exclusion (the Starnes procedure) could result in about 80% survival at 5 years; however, these patients require single ventricle circulation. When considering biventricular management, the higher PVR seen in neonates mandates a higher degree of TV competency after repair and makes the use of a superior cavopulmonary connection impractical [26]. The presence of anatomic valvular PS or PA increased RV pressure, or it led to regurgitation if a transannular patch was placed. These features suggested that the patient could not survive solely with a well-repaired TV, if RV function was not good enough.
Staged Biventricular Repair To overcome the difficulty, our idea came from the experience of PA and intact ventricular septum (also called hypoplastic right heart syndrome). A “staged” biventricular approach for these patients had excellent results [27, 28]. Most patients with the potential for complete biventricular repair reached complete biventricular repair beyond infancy, as shown by the Congenital Heart Surgeons Society (CHSS) study [29]. This concept takes a similar approach with Ebstein anomaly, to overcome the difficulty of “complete” biventricular circulation in neonatal periods. The fenestrated ASD can help to maintain systemic cardiac output in the setting of RV failure, at the expense of oxygen saturation. If the right ventricle cannot generate enough pulmonary blood flow, adding a BT shunt can improve pulmonary blood flow. In our series, when the pulmonary arteries were smaller, then there was a greater chance of postoperative cyanosis or RV failure requiring a shunt. The ASD should be ready for an adequate right-to-left shunt if a BT shunt is added. This physiology became “partial” biventricular circulation. The right ventricle is part “in series” and part “in parallel” with the left ventricle. As RV function improved, more and more systemic venous blood went through right ventricle, and less and less went through the ASD. When RV function or PVR improved, the shunt was no longer required, and the ASD was not needed in the end. Although the fenestrated ASD and shunt can help overcome high PVR and inadequate RV function, good TV repair is still essential to decrease heart distention. A systemic-pulmonary shunt in the presence of severe pulmonary regurgitation, in addition to TR, results in a “circular shunt,” which is difficult to manage medically. Therefore a higher degree of TV competency is needed in the presence of pulmonary regurgitation, especially when a shunt is placed. The one death among our patients suggested that earlier redo TVP is important if significant TR is found. Our strategy (TVP, RA reduction, repair of associated defects, fenestrated ASD, and a shunt if needed) can maintain the potential of biventricular circulation in most patients with Ebstein anomaly. The shunt and the ASD can be closed through cardiac catheterization if they are no longer needed. The management of residual PS or
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Right Ventricular Outflow Tract Reconstruction Knott-Craig and colleagues [12] reported a series of 32 neonates with Ebstein anomaly, and the mortality rate for patients with anatomic PA was 40% (8 of 20). The introduction of an RV-PA homograft conduit improved the results significantly, with a 16% (1 of 6) mortality rate in the highest-risk group. However, neither the homograft nor the bovine jugular venous conduit (Contegra, Medtronic, Minneapolis, MN) is available in Taiwan. We think that a competent pulmonary valve in the presence of Ebstein anomaly and PA, during the neonatal high PVR stage, could further improve the result of TVP and biventricular repair.
Fate of Atrialized Right Ventricle Most adults or children with Ebstein anomaly have received TVP with plication of atrialized right ventricle [17, 19]. In the patients with unrepaired Ebstein anomaly, atrialized right ventricle dilated and became a thin and noncontractile part of the right ventricle. We found that the atrialized part of the right ventricle did not dilate after TVP; however, mild paradoxic septal contraction could be found in this part of the interventricular septum. This finding suggests that the atrialized right ventricle, as delineated by the abnormal insertion of the TV septal leaflet, could have had an intrinsic abnormality [6]. We believe that the progressive dilatation and interstitial fibrosis of atrialized right ventricle could be halted by earlier repair of Ebstein anomaly, and RV function could be preserved. Longer follow-up is needed to see whether the right ventricle could further remodel and the positive impact could be sustained.
Study Limitations This study is limited by the small number of cases and the heterogeneity of the anatomic details. Ebstein anomaly has a highly variable presentation, so there might not be a single surgical strategy to fit all patients. For this complex group of patients, more experience is needed to help understand the disease and to improve the outcome of this fatal disease.
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Conclusions TVP incorporating the principle of cone reconstruction is an acceptable surgical strategy in severe neonatal Ebstein anomaly. Fenestrated ASD and systemic-pulmonary shunt can help to overcome RV failure and high PVR in the neonatal period. This surgical strategy has good survival outcomes and preserves the possibility for complete biventricular repair. This work was supported in part by Taiwan Ministry of Science and Technology grant number MOST 104-2314-B-002-203.
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regurgitation will be easier as the child grows up and achieves better physiologic status. Sano and associates [13] reported a series of 12 neonates or infants with Ebstein anomaly and PS or PA. These investigators proposed a biventricular repair for patients with acceptable RV function with six primary biventricular repairs and another two staged repairs after a BT shunt. The proposed criteria for biventricular repair included TR greater than 30 mm Hg with antegrade main pulmonary artery flow. Our 5 patients with PS or PA did not fulfill these criteria proposed by Sano and colleagues [13], but we still had 80% (4 of 5 patients) survive. We think that the criteria put forth by Sano and colleagues [13] are good references for a “primary” biventricular repair strategy, and if patients do not fit all criteria, then “staged” biventricular repair or even single ventricle repair should be considered.
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17. Dearani JA, Said SM, O’Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein’s malformation: lessons learned with cone reconstruction. Ann Thorac Surg 2013;95: 220–8. 18. da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 2007;133:215–23. 19. da Silva JP, da Silva Lda F. Ebstein’s anomaly of the tricuspid valve: the cone repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2012;15:38–45. 20. Li X, Wang SM, Schreiber C, et al. More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol 2016;206:131–7. 21. Ling YW, Shih JC, Wu ET, Chen SJ, Chen YS, Huang SC. Surgical correction for a neonate with Ebstein’s anomaly associated with Tetralogy of Fallot. Ann Thorac Surg. In Press. 22. Chang YM, Wang JK, Chiu SN, et al. Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort. Eur J Pediatr 2009;168:685–90. 23. Knott-Craig CJ, Overholt ED, Ward KE, Razook JD. Neonatal repair of Ebstein’s anomaly: indications, surgical technique,
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24. 25.
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28.
29.
and medium-term follow-up. Ann Thorac Surg 2000;69: 1505–10. Reemtsen BL, Fagan BT, Wells WJ, Starnes VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 2006;132:1285–90. Reemtsen BL, Polimenakos AC, Fagan BT, Wells WJ, Starnes VA. Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2007;134:1406–10; discussion 1410–2. Raju V, Dearani JA, Burkhart HM, et al. Right ventricular unloading for heart failure related to Ebstein malformation. Ann Thorac Surg 2014;98:167–73; discussion 173–64. Huang SC, Ishino K, Kasahara S, Yoshizumi K, Kotani Y, Sano S. The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa. J Thorac Cardiovasc Surg 2009;138:1160–6. Sano S, Ishino K, Kawada M, Fujisawa E, Kamada M, Ohtsuki S. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg 2000;70:1501–6. Ashburn DA, Blackstone EH, Wells WJ, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 2004;127:1000–7; discussion 1007–8.
DISCUSSION DR T. K. SUSHEEL KUMAR (Memphis, TN): First of all, I want to congratulate you and your team on an excellent presentation, and thank you for sending us a copy of your manuscript and the presentation well ahead of time. As we all know, Ebstein’s anomaly is a rare congenital heart disease and management in the symptomatic neonate can be a medical and a surgical challenge. We also do know that Ebstein’s anomaly is just not an isolated disease of the tricuspid valve. It is also a disease of the right ventricular myocardium, often with significant atrialization of the right ventricle and small functional right ventricle. The condition carries significant mortality in the symptomatic neonates and is uniformly fatal without timely surgical intervention when conservative measures fail. The surgical approach to the symptomatic neonate has varied widely between an aggressive biventricular repair as advocated by Dr Christopher Knott-Craig and a single ventricle approach as pioneered by Dr Vaughn Starnes. Now, the critics of the biventricular approach will point out that the right ventricle may not have sufficient capacity to handle the cardiac output, while critics of the single ventricle approach will argue that by causing functional tricuspid atresia, one has taken away the opportunity for the right ventricle to grow. A recent survey in the United States shows the wide variation in practice with regard to a symptomatic neonate. Against this background, your concept of a staged biventricular management adds a new approach to a very difficult problem. Your biventricular approach, as you mentioned in the manuscript, is based on the concept of management of pulmonary atresia with intact ventricular septum and has worked well for your patients. Now, before going to my questions, I have to say that by combining tricuspid valve replacement with relief of pulmonary outflow tract obstruction and the addition of atrial septal defect (ASD) and Blalock-Taussig shunt, you are reducing the burden on the right ventricle. In a way, you are giving the right ventricle an opportunity to declare itself. If the right ventricle grows and begins to take on more of the cardiac output, the patient can head toward a two ventricle repair. Maybe that could be achieved in the cardiac catheterization lab.
Now, as against that, if the right ventricle does not grow, the patient could be taken along the single ventricle pathway, including upsizing of shunt if needed, Glenn or Fontan. So I think those are the merits of your approach. Now, although the concept is very attractive, there are some inherent flaws which I would like to point out before going into the questions. The right ventricular myocardium in the pulmonary atresia intact ventricular septum, you will agree with me, is completely different from the myocardium in an Ebstein’s patient. So it would be wrong to compare the growth potentials of the myocardium in two different conditions. A dilated right ventricle with residual tricuspid regurgitation and possible pulmonary regurgitation can compromise early function. However, as your results show, some of these fears are unfounded. My questions are here. The first one is regarding the cone technique. The performance of a cone technique in a neonate can be technically challenging. As you know, the original technique was described by Dr da Silva and modified by Dr Joe Dearani by addition of a pericardial patch. Did you have to do that on any of your patients? Did you use the classic technique or did you use the modified technique? DR HUANG: Indeed, I started to do this kind of repair before I saw the paper by da Silva. Indeed, I got the experience from Professor Sano. And I did not have any experience of adding a pericardial patch, because in these 7 patients we could make a competent tricuspid valve by its own tissue. DR KUMAR: Thank you. My second question is about the association of pulmonary stenosis or atresia. As we know, sometimes up to half of the symptomatic patients with Ebstein’s anomaly can have functional or anatomic pulmonary atresia. Now, as our own group showed, results with the biventricular approach can be poor unless you have a valved conduit, and our own mortality with a transannular patch was 40% and this was reduced to 16% once we started using a valved conduit. And as you know, this may be related to the addition of extra volume on
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DR HUANG: In our country, we do not have a small homograft and we do not have the Contegra for reconstruction of the right ventricular outflow tract. We only have the Gore-Tex membrane with a handmade monocuspid method to make a pulmonary valve. So it becomes an issue for dealing with a patient requiring a pulmonary valve. It is quite a challenge in our hospital. DR KUMAR: I understand that. The third question relates to the conclusion that you made in the last slide. It says that 6 of your 7 patients achieved biventricular repair, but in 2 of your patients, as mentioned in the manuscript, the ASD is still large and the flow was right to left. I think it may be premature to declare successful biventricular repair on patients unless their ASD closed or the flow across the ASD is predominantly left to right. Do you have anything to say about that? DR HUANG: Yes, I agree that it is not completed yet, but we anticipate completing it. DR KUMAR: And the last question that I have relates to the demonstration of the video. I think a few of us had the opportunity to view the video 2 days back. I have to congratulate you once again on your excellent approach and the excellent quality of the video presentation. You combined your tetralogy of Fallot repair with an Ebstein’s, and that was very much appreciated. But as I pointed out that evening I would like to reiterate that you should also pay attention to whether you can preserve the pulmonary valve or not. If you cannot preserve the pulmonary valve, I think that you may not have had such a good result. Do you agree with me on that?
925
DR HUANG: I understand in the neonatal cases, preserving a competent pulmonary valve and tricuspid valve is very important, in contrast to that in the late infancy or the adolescent. So if the pulmonary valve could not be competent, we need a very competent tricuspid valve after repair. This is the key for survival if we do not do a Starnes operation or single ventricle palliation. We do agree. DR KUMAR: Dr Huang, I want to congratulate you once again. I enjoyed reading the manuscript. I learned a lot from reading your manuscript. Thank you very much. DR BRIAN L. REEMTSEN (Los Angeles, CA): I was actually with Vaughn and wrote up most of his initial data on the Starnes technique, but my thoughts and my practice have evolved. One of the biggest reasons is that when these babies are born, even when they have wall-to-wall hearts, a lot of times if they do not have pulmonary stenosis, they can be medically treated and they can leave the hospital. I am trying to find a reason to operate on these kids, and they are doing well and they are getting out of the intensive care unit. I just want to know what were these severe symptoms these kids were having who just had severe tricuspid valve regurgitation and no pulmonary stenosis, because in our hospital, most of those patients, the grand majority, leave the hospital. DR HUANG: The same. In our hospital if the patient does not have a ventricular septal defect and severe tricuspid regurgitation or pulmonary valve stenosis or atresia, most of them were discharged without operation. The heart becomes smaller and they are discharged from the hospital without mortality. These particular patients I operated were because of severe tricuspid regurgitation and the functional pulmonary atresia, or with the Great Ormond Street index above 1. It predicts 100% mortality, so we go on to surgery. DR REEMTSEN: What I am saying is if they are on drips, they cannot get off the ventilator, what is making you operate? DR HUANG: No. All the patients could not separate from the ventilator, so we do the surgery. If the patient was separated from ventilator, the patient could be discharged.
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the right ventricle or creation of a circular shunt. As we also realize the only fatality that you pointed out in your group of patients had a transannular patch and possibly resulted in early volume overload. In your manuscript you mention among the limitations that there are no homografts available in your country. That is mentioned in the manuscript. Did you consider any alternatives like a valved CorMatrix or a valved polytetrafluoroethylene graft?
HUANG ET AL REPAIRING NEONATAL EBSTEIN’S ANOMALY
Background. Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. Methods. Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n [ 5), and a systemic-pulmonary shunt was added if needed (n [ 3). Results. All patients presented with intractable heart failure or severe cyanosis requiring mechanical
ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n [ 2) and tetralogy of Fallot (n [ 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years). Conclusions. Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.
M
Conversely, biventricular repair was proposed for symptomatic neonates with Ebstein anomaly by KnottCraig and colleagues [10–12] and some limited case reports from other centers [13–15]. The technique of tricuspid valvuloplasty (TVP) for Ebstein anomaly evolved over time [16, 17]. The introduction of cone reconstruction [18, 19] significantly improved surgical results and has become a widely accepted technique for children and adults [12, 14, 20], but TVP has not been carried out much in neonates. The difficulty of neonatal Ebstein anomaly repair lies not only in the delicate surgical techniques for repairing a neonatal TV, but also in overcoming the high pulmonary vascular resistance (PVR) in neonatal physiology. Since 2007 we have performed TV repair for neonatal Ebstein anomaly. Here we describe our surgical strategy aiming for biventricular repair.
anagement of a neonate with symptomatic Ebstein anomaly is a very challenging situation, and it carries a high risk of early death [1–6]. Tricuspid valve (TV) repair for symptomatic neonatal Ebstein anomaly is reportedly an extremely high-risk procedure, especially in patients with Ebstein anomaly and right ventricular (RV) outflow obstruction [4, 7, 8]. In 1991 the Starnes operation was introduced for severe neonatal Ebstein anomaly. This procedure involved the univentricularization of the heart by TV closure using a fenestrated patch and a modified Blalock-Taussig shunt (BT shunt) [9]. The Starnes operation as a method of single ventricle palliation is advocated for relatively simple operations with acceptable survival rates for neonates or young infants with Ebstein anomaly. Accepted for publication Jan 18, 2017. Presented at the Sixty-third Annual Meeting of the Southern Thoracic Surgical Association, Naples, FL, Nov 9–12, 2016. Address correspondence to Dr Huang, Department of Surgery, National Taiwan University Hospital, 7 Chung-Shan South Rd, Taipei 100, Taiwan; email: [email protected].
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier Inc.
(Ann Thorac Surg 2017;104:917–25) Ó 2017 by The Society of Thoracic Surgeons
Patients and Methods Between 2007 and 2016, 7 severely symptomatic neonates (n ¼ 5) or young infants (n ¼ 2) with Ebstein anomaly 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2017.01.081
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Surgical Strategy Toward Biventricular Repair for Severe Ebstein Anomaly in Neonates and Infancy
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underwent TV repair (TVP) operations at the National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. We did not have experience with the Starnes operation in this cohort. The patients with pulmonary atresia (PA), intact ventricular septum, severe hypoplastic right ventricle, and small tricuspid annulus (Z score <-2) with Ebstein anomaly were managed by single ventricle reconstruction according to the TV Z score. The patients’ medical records and operative notes were reviewed. This study was approved by the Institutional Review Board of National Taiwan University Hospital, and informed consent was waived.
Surgical Technique The operation included TVP, right atrial (RA) reduction, management of RV outflow, and atrial septal defect (ASD) closure or fenestration. The surgical procedure was performed through a standard median sternotomy and cardiopulmonary bypass. The patent ductus arteriosus was ligated after institution of cardiopulmonary bypass. TV repair was performed with the aorta cross-clamped. The basic principle follows the cone reconstruction technique [18]. The technique is aimed at complete surgical delamination and recruitment of all undelaminated leaflet tissue. The anterior leaflet was incised along the annulus and carefully detached from its adherence to the RV free wall, and the most important papillary muscle and its chordae were preserved. The posterior leaflet and the septal leaflets were also detached from their abnormal insertion sites. Then the edge of the TV apparatus was reattached to the true atrial-ventricular TV annulus. The annulus size was determined according to body weight. Saline testing was performed, and sometimes additional annular plication stitches were applied (Fig 1). The associated ventricular septal defect (VSD) was repaired with a Dacron patch (Bard Sauvage Filamentous Knitted Polyester Fabric, Tempe, AZ) in the usual manner. For the particular patient with tetralogy of Fallot, a patch was placed on the RV outflow tract (RVOT) to Fig 1. (A) The tricuspid valve apparatus was detached from the annulus and from its adherence to the right ventricular wall. (B) The tricuspid valve was reattached to the true annulus, thus making a competent valve at its normal position (patient 4).
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relieve the infundibular stenosis after TV repair and VSD repair [21]. This atrialized RV was not plicated. The ASD was closed in 2 patients with Ebstein anomaly and VSD without pulmonary stenosis (PS), and fenestration was kept for the other patients with PS. One piece of RA wall (approximately 2 cm wide) was excised before closure, so the size of the dilated right atrium was reduced. For the 5 patients with PS, the pulmonary valve was preserved in 3 of them because the valve diameter was approximately 5 mm by intraoperative measurement. The other 2 patients with diminutive pulmonary valves received transannular patches. After trials to wean the patients from cardiopulmonary bypass, 3 patients received additional BT shunts for cyanosis. We routinely delayed sternum closure until the patients were in stable condition. Inotropic agents, including low-dose dopamine, milrinone, and sometimes epinephrine, were used for postoperative RV failure, and inhalation nitric oxide was administered to decrease PVR.
Data Analysis Descriptive statistics were used. Data are expressed as the mean standard deviation or the median and range as appropriate. The end point of the study was early death, defined as death before hospital discharge or within 30 days of the surgical procedure.
Results The patients’ cases are summarized in Table 1.The mean weight of the patients at the time of operation was 2.9 0.7 kg, ranging from 2.0 to 3.7 kg, and the mean age at the time of surgical intervention was 31 30 days. The diagnosis had been made prenatally in 5 (71%) of the patients. Two patients had associated VSD, and the indication for operation was intractable heart failure. One patient had associated tetralogy of Fallot and presented with severe heart failure and severe cyanosis. The other 4 patients had PS or functional PA and presented with severe cyanosis, with 1 patient requiring immediate
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Table 1. Demographic Features, Echocardiographic Findings, and Clinical Presentation of the 7 Patients With Ebstein Anomaly CXR
Echocardiographic Findings
TV Downward Patient Age Displacement No. (days) BW (kg) C/T Ratio (mm) 23
3.1
0.76
7.3
2
102
3.4
0.7
12.8
3
18
2.7
0.84
6.9
4
36
3.4
0.88
16.3
5
6
2
0.91
6
11
2.1
0.83
7
27
3.7
0.79
Celermajer Index [2]
RVOT Flow
Pulmonary Annulus Carpentier (mm) Classification
Associated Lesions
Preoperative Condition
Moderate (51 mm Hg) Severe (58 mm Hg)
0.7
No PS
10
C
VSD, perimembranous NIPPV, heart failure
1.01
No PS
8
C
VSD, muscular trabecular, aberrant RSCA
Severe (34 mm Hg) Severe (8 mm Hg)
0.98
Poor antegrade flow, moderate PR No antegrade flow
10
B–C
Valvular PS
7.1
C–D
9.1
Severe (34 mm Hg)
1.5
No antegrade flow, PR(þ)
6.5
C
Functional pulmonary atresia Severe PS
5.7
Severe (90 mm Hg) Severe (14 mm Hg)
1.34
Scant antegrade flow
6.3
B
Critical PS
1.27
PS, 10 mm Hg
5.3
C
Tetralogy of Fallot, Ventilator, cyanosis, heart mild RV hypoplasia failure
15
1.85
Status after PDA ligation, prematurity, acute renal failure, subglottic stenosis, Ventilator, heart failure Ventilator, cyanosis Cyanosis after shunt, ventilator, heart failure Prematurity, hydrops fetalis, ECMO immediately after birth Ventilator, iNO, shock
BW ¼ body weight; C/T ratio ¼ cardiothoracic ratio; CXR ¼ chest roentgenogram; ECMO ¼ extracorporeal membrane oxygenation; iNO ¼ inhalation nitric oxide; positive pressure ventilation; PDA ¼ patent ductus arteriosus; PR ¼ pulmonary regurgitation; PS ¼ pulmonary stenosis; RSCA ¼ right subclavian artery; RVOT ¼ right ventricular outflow tract; TR ¼ tricuspid regurgitation; TV ¼ tricuspid valve; VSD ¼ ventricular septal defect.
NIPPV ¼ noninvasive RV ¼ right ventricular;
HUANG ET AL REPAIRING NEONATAL EBSTEIN’S ANOMALY
1
TR Severity (Pressure Gradient)
919
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920
HUANG ET AL REPAIRING NEONATAL EBSTEIN’S ANOMALY
extracorporeal membrane oxygenation (ECMO) support after birth. Two patients underwent surgical procedures before TVP; 1 patient had ligation of the patent ductus arteriosus for heart failure, whereas the other patient received a BT shunt for functional PA and severe cyanosis. Neither patient could be separated from ventilator support before the TVP operation. The operative details and outcomes are summarized in Table 2.
Mortality One death occurred (patient 6). This 2.1-kg girl had a prenatal diagnosis, and she received high-frequency oscillatory ventilation, inhalation nitric oxide, dopamine, and dobutamine for desaturation and shock. She was referred to our hospital, and an emergency operation was performed. Ebstein anomaly with a critical anatomic valvular stenosis (no antegrade flow noted) was found. She received TVP, ASD fenestration, RA reduction, an RVOT patch, and a 3-mm shunt. On the second postoperative day, ECMO was begun for worsening oxygen saturation. The patient was able to be weaned from ECMO with her oxygen saturation at approximately 80% to 90% after we changed the BT shunt from a 3-mm graft to a 3.5-mm graft. However, 2 months later, an episode of severe desaturation and shock required another course of ECMO. Severe tricuspid regurgitation (TR) was noted, and the posterior leaflet was found to be detached as a result of torn stitches. Despite repeat TVP, shunt division, and RVOT reconstruction, she did not recover from renal failure and multiorgan dysfunction and died 1 month after the second operation.
Ventilator Use The median time to extubation was 19 days. Generally, the saturation level gradually improved as the PVR decreased or RV function improved after the surgical procedure in the first postoperative week. One patient (patient 2) struggled for 3 months before accepting operation for Ebstein anomaly with severe TR and VSD. She had subglottic stenosis caused by repeated preoperative intubation and required postoperative tracheostomy. All the other patients were successfully extubated. No patients required supplement oxygen after discharge.
Extracorporeal Membrane Oxygenation One patient (patient 5) received ECMO preoperatively. She had a prenatal diagnosis of Ebstein anomaly with severe TR, pulmonary regurgitation, and hydrops fetalis. She was born by cesarean delivery and had profound edema, cyanosis, and bradycardia. We performed ECMO and patent ductus arteriosus ligation immediately. There was virtually no antegrade pulmonary blood flow, and the main and bilateral pulmonary arteries were less than 2 mm in diameter. After her edematous status improved, we performed TVP, pulmonary valve commissurotomy with a small RVOT patch, and a right BT shunt (3 mm) when she was 6 days old. ECMO was maintained after the
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operation for 5 days. Because of the persistent small right pulmonary artery with juxtashunt stenosis, balloon dilatation of the right pulmonary artery was performed. After a prolonged stay in the intensive care unit, her saturation gradually improved, and she was eventually extubated 92 days postoperatively.
Follow-Up and Reintervention Six patients were discharged with a median follow-up duration of 4.5 years (range, 0.8 to 9.9 years). All 6 patients are currently surviving. The TR was mild in 5 patients and moderate in 1 patient. For the patients with PS (n ¼ 4), 1 had residual moderate PS, and the other 3 had PS well relieved with mild pulmonary regurgitation.
Fate of Fenestrated Atrial Septal Defect The first 2 patients (patients 1 and 2) with VSD had a successful repair procedure without any right-to-left shunt. Patient 4 already had his shunt occluded and a tiny ASD. Patient 6, with tetralogy of Fallot, had a left-toright shunt through an ASD. The 4 patients had complete biventricular circulation, and only regular follow-up for long-term TV function was needed. Patient3 had residual PS and moderate TR and will possibly require reintervention on the pulmonary valve and TV if the condition worsens. Patient 5 had residual right pulmonary artery stenosis after balloon dilatation. We plan to carry out surgical pulmonary arterioplasty and closure of the shunt in the future. These 2 patients (patients 3 and 5) had right-to-left shunts through the fenestrated ASD. In summary, for the 6 survivors, 4 patients had separate biventricular circulations achieved, and 2 remained in mixed circulation (with right-to-left shunt through the ASD). We anticipate that the 2 aforesaid patients can achieve complete biventricular circulation by closure of the ASD. The atrialized right ventricle did not dilate during the follow-up period, and it became part of the functioning right ventricle after TVP in all the survivors. However, minimal paradoxic septal contraction was still noted on this part of the interventricular septum (Fig 2).
Comment In the cohort, we performed TVP in 7 neonates with severe Ebstein anomaly; 6 (85%) patients survived and achieved biventricular circulation. The result was similar to that of the largest series reported, with an early survival rate of 78.1% (25 of 32) for biventricular repair in neonates and infants with Ebstein anomaly [1].
Indications for Early Surgical Intervention: Associated Ventricular Septal Defect or Pulmonary Stenosis or Atresia Not all babies born with Ebstein anomaly require surgical intervention in early infancy. The Toronto group [7] reported that 75% of these patients did not require
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Table 2. Operative Details and Outcomes
1 2 3
4 5
6
TVP, fenestrated ASD, Functional PA, shunt 3.5 mm mild PS: 5 mm TVP, RVOT patch, True PS, 3 mm fenestrated ASD, BT shunt 3 mm Critical valvular PS TVP, RVOT patch, fenestrated ASD, shunt 3 mm TVP, tetralogy of Fallot Tricuspid, mild repair, fenestrated PS, 5 mm ASD
Follow-Up (y)
146 (101)
7
Survival
9.9
170 (93)
28
Survival
6.5
175 (118)
10
Survival
4.8
106 (69)
24
Survival
4.3
193 (50)
92
Survival
2.5
101 (53)
X
.
152 (124)
14
Postoperative ECMO, change to 3.5 mm shunt, Survival
0.8
TR (PG)
Pulmonary Valve
Mild TR Normal 22 mm Hg Mild TR Normal (24 mm Hg) Mild TR Moderate PS, (34 mm Hg) PG: 36 mm Hg, mild PR Moderate TR Mild PR (32 mm Hg) Mild TR Mild PR (26 mm Hg) .
Mild TR No PS (18 mm Hg)
ASD ¼ atrial septal defect; AXC ¼ aorta cross-clamp time; BT ¼ Blalock-Taussig; CPB ¼ total duration of cardiopulmonary bypass; L / R ¼ left-to-right; PA ¼ pulmonary atresia; PG ¼ pressure gradient; PR ¼ pulmonary regurgitation; PS ¼ pulmonary stenosis; outflow tract; TR ¼ tricuspid regurgitation; TVP ¼ tricuspid valvuloplasty; VSD ¼ ventricular septal defect; X ¼ indicate mortality.
.
ASD
Others
Closed
.
Closed
Tracheostomy for subglottic stenosis .
R/L
Tiny R/L shunt .
Shunt closed at 11 months old Right PA stenosis, shunt partially occluded Death 3 months postoperatively (TR)
L / R shunt
ECMO ¼ extracorporeal membrane oxygenation; R / L ¼ right-to-left; RVOT ¼ right ventricular
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7
TVP, VSD repair, Normal ASD closure TVP, VSD repair, Normal ASD closure TVP, fenestrated ASD PS(þ) valvular dysplasia
Outcome
921
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Ventilator Pulmonary Valve CPB (AXC) (Postoperative Patient Operative Procedure (Operative Finding) (min) Days)
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Fig 2. (A) Preoperative and (B) postoperative echocardiograms. The downwardly displaced tricuspid valve was restored to its normal position with a normal appearance. The atrialized right ventricle is shown (arrows) (patient 4).
operations at 1 month old, and similar results were shown in our previous reports [22]. In the presence of a significant VSD, the right ventricle continues to operate at high pressure, thereby magnifying the degree of TR and additional volume overload. Moreover, the presence of a left ventricle (through VSD and TR)–to-RA shunt could further compromise systemic cardiac output and increase systemic venous pressure, thus resulting in intractable heart failure. The presence of PS or PA added a pressure load in addition to the decreased volume of functioning RV. The small functioning RV therefore could not generate enough blood flow to the pulmonary circulation, even if the PVR decreased. In this series, all 7 patients had VSD, PS, or PA. This finding is similar to those of other reported series of neonatal Ebstein anomaly [10, 13]. In the same cohort, other patients with Ebstein anomaly without VSD, PS, or PA did not receive operations in their infancy, and all survived at our institution.
Choice of Operation Severely symptomatic neonates with Ebstein anomaly have an early mortality rate of nearly 100% without surgical intervention [2, 6, 23]. It is still unclear which surgical strategy is the best option for these critically ill neonates. A shunt-only option for patients with Ebstein anomaly with cyanosis or PA has been proposed [5]. However, we had poor results (67% mortality rate) in neonatal Ebstein anomaly in the era before 2007 [22], so we changed this policy. Starnes and colleagues [9, 24, 25] showed that the outcome of TV exclusion (the Starnes procedure) could result in about 80% survival at 5 years; however, these patients require single ventricle circulation. When considering biventricular management, the higher PVR seen in neonates mandates a higher degree of TV competency after repair and makes the use of a superior cavopulmonary connection impractical [26]. The presence of anatomic valvular PS or PA increased RV pressure, or it led to regurgitation if a transannular patch was placed. These features suggested that the patient could not survive solely with a well-repaired TV, if RV function was not good enough.
Staged Biventricular Repair To overcome the difficulty, our idea came from the experience of PA and intact ventricular septum (also called hypoplastic right heart syndrome). A “staged” biventricular approach for these patients had excellent results [27, 28]. Most patients with the potential for complete biventricular repair reached complete biventricular repair beyond infancy, as shown by the Congenital Heart Surgeons Society (CHSS) study [29]. This concept takes a similar approach with Ebstein anomaly, to overcome the difficulty of “complete” biventricular circulation in neonatal periods. The fenestrated ASD can help to maintain systemic cardiac output in the setting of RV failure, at the expense of oxygen saturation. If the right ventricle cannot generate enough pulmonary blood flow, adding a BT shunt can improve pulmonary blood flow. In our series, when the pulmonary arteries were smaller, then there was a greater chance of postoperative cyanosis or RV failure requiring a shunt. The ASD should be ready for an adequate right-to-left shunt if a BT shunt is added. This physiology became “partial” biventricular circulation. The right ventricle is part “in series” and part “in parallel” with the left ventricle. As RV function improved, more and more systemic venous blood went through right ventricle, and less and less went through the ASD. When RV function or PVR improved, the shunt was no longer required, and the ASD was not needed in the end. Although the fenestrated ASD and shunt can help overcome high PVR and inadequate RV function, good TV repair is still essential to decrease heart distention. A systemic-pulmonary shunt in the presence of severe pulmonary regurgitation, in addition to TR, results in a “circular shunt,” which is difficult to manage medically. Therefore a higher degree of TV competency is needed in the presence of pulmonary regurgitation, especially when a shunt is placed. The one death among our patients suggested that earlier redo TVP is important if significant TR is found. Our strategy (TVP, RA reduction, repair of associated defects, fenestrated ASD, and a shunt if needed) can maintain the potential of biventricular circulation in most patients with Ebstein anomaly. The shunt and the ASD can be closed through cardiac catheterization if they are no longer needed. The management of residual PS or
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Right Ventricular Outflow Tract Reconstruction Knott-Craig and colleagues [12] reported a series of 32 neonates with Ebstein anomaly, and the mortality rate for patients with anatomic PA was 40% (8 of 20). The introduction of an RV-PA homograft conduit improved the results significantly, with a 16% (1 of 6) mortality rate in the highest-risk group. However, neither the homograft nor the bovine jugular venous conduit (Contegra, Medtronic, Minneapolis, MN) is available in Taiwan. We think that a competent pulmonary valve in the presence of Ebstein anomaly and PA, during the neonatal high PVR stage, could further improve the result of TVP and biventricular repair.
Fate of Atrialized Right Ventricle Most adults or children with Ebstein anomaly have received TVP with plication of atrialized right ventricle [17, 19]. In the patients with unrepaired Ebstein anomaly, atrialized right ventricle dilated and became a thin and noncontractile part of the right ventricle. We found that the atrialized part of the right ventricle did not dilate after TVP; however, mild paradoxic septal contraction could be found in this part of the interventricular septum. This finding suggests that the atrialized right ventricle, as delineated by the abnormal insertion of the TV septal leaflet, could have had an intrinsic abnormality [6]. We believe that the progressive dilatation and interstitial fibrosis of atrialized right ventricle could be halted by earlier repair of Ebstein anomaly, and RV function could be preserved. Longer follow-up is needed to see whether the right ventricle could further remodel and the positive impact could be sustained.
Study Limitations This study is limited by the small number of cases and the heterogeneity of the anatomic details. Ebstein anomaly has a highly variable presentation, so there might not be a single surgical strategy to fit all patients. For this complex group of patients, more experience is needed to help understand the disease and to improve the outcome of this fatal disease.
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Conclusions TVP incorporating the principle of cone reconstruction is an acceptable surgical strategy in severe neonatal Ebstein anomaly. Fenestrated ASD and systemic-pulmonary shunt can help to overcome RV failure and high PVR in the neonatal period. This surgical strategy has good survival outcomes and preserves the possibility for complete biventricular repair. This work was supported in part by Taiwan Ministry of Science and Technology grant number MOST 104-2314-B-002-203.
References 1. Boston US, Goldberg SP, Ward KE, et al. Complete repair of Ebstein anomaly in neonates and young infants: a 16-year follow-up. J Thorac Cardiovasc Surg 2011;141:1163–9. 2. Celermajer DS, Cullen S, Sullivan ID, Spiegelhalter DJ, Wyse RK, Deanfield JE. Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 1992;19:1041–6. 3. Freud LR, Escobar-Diaz MC, Kalish BT, et al. Outcomes and predictors of perinatal mortality in fetuses with Ebstein anomaly or tricuspid valve dysplasia in the current era: a multicenter study. Circulation 2015;132:481–9. 4. Shinkawa T, Polimenakos AC, Gomez-Fifer CA, et al. Management and long-term outcome of neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2010;139:354–8. 5. Bove EL, Hirsch JC, Ohye RG, Devaney EJ. How I manage neonatal Ebstein’s anomaly. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2009;12:63–5. 6. Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE. Morbid anatomy in neonates with Ebstein’s anomaly of the tricuspid valve: pathophysiologic and clinical implications. J Am Coll Cardiol 1992;19:1049–53. 7. Wertaschnigg D, Manlhiot C, Jaeggi M, et al. Contemporary outcomes and factors associated with mortality after a fetal or neonatal diagnosis of Ebstein anomaly and tricuspid valve disease. Can J Cardiol 2016;32:1500–6. 8. Pizarro C. The challenging quest of neonatal Ebstein management. J Thorac Cardiovasc Surg 2016;152:522–3. 9. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein’s anomaly appearing in the neonate: a new surgical approach. J Thorac Cardiovasc Surg 1991;101: 1082–7. 10. Knott-Craig CJ, Goldberg SP, Ballweg JA, Boston US. Surgical decision making in neonatal Ebstein’s anomaly: an algorithmic approach based on 48 consecutive neonates. World J Pediatr Congenit Heart Surg 2012;3:16–20. 11. Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS, Razook JD. Repair of Ebstein’s anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up. Ann Thorac Surg 2002;73:1786–92; discussion 1792–83. 12. Knott-Craig CJ, Kumar TK, Arevalo AR, Joshi VM. Surgical management of symptomatic neonates with Ebstein’s anomaly: choice of operation. Cardiol Young 2015;25:1119–23. 13. Sano S, Fujii Y, Kasahara S, et al. Repair of Ebstein’s anomaly in neonates and small infants: impact of right ventricular exclusion and its indications. Eur J Cardiothorac Surg 2014;45:549–55; discussion 555. 14. Mizuno M, Hoashi T, Sakaguchi H, et al. Application of cone reconstruction for neonatal Ebstein anomaly or tricuspid valve dysplasia. Ann Thorac Surg 2016;101:1811–7. 15. Sata S, Murin P, Hraska V. Cone reconstruction of Ebstein’s anomaly in a neonate. Ann Thorac Surg 2012;94:e99–100; discussion e100. 16. Hetzer R, Hacke P, Javier M, et al. The long-term impact of various techniques for tricuspid repair in Ebstein’s anomaly. J Thorac Cardiovasc Surg 2015;150:1212–9.
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regurgitation will be easier as the child grows up and achieves better physiologic status. Sano and associates [13] reported a series of 12 neonates or infants with Ebstein anomaly and PS or PA. These investigators proposed a biventricular repair for patients with acceptable RV function with six primary biventricular repairs and another two staged repairs after a BT shunt. The proposed criteria for biventricular repair included TR greater than 30 mm Hg with antegrade main pulmonary artery flow. Our 5 patients with PS or PA did not fulfill these criteria proposed by Sano and colleagues [13], but we still had 80% (4 of 5 patients) survive. We think that the criteria put forth by Sano and colleagues [13] are good references for a “primary” biventricular repair strategy, and if patients do not fit all criteria, then “staged” biventricular repair or even single ventricle repair should be considered.
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17. Dearani JA, Said SM, O’Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein’s malformation: lessons learned with cone reconstruction. Ann Thorac Surg 2013;95: 220–8. 18. da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 2007;133:215–23. 19. da Silva JP, da Silva Lda F. Ebstein’s anomaly of the tricuspid valve: the cone repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2012;15:38–45. 20. Li X, Wang SM, Schreiber C, et al. More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol 2016;206:131–7. 21. Ling YW, Shih JC, Wu ET, Chen SJ, Chen YS, Huang SC. Surgical correction for a neonate with Ebstein’s anomaly associated with Tetralogy of Fallot. Ann Thorac Surg. In Press. 22. Chang YM, Wang JK, Chiu SN, et al. Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort. Eur J Pediatr 2009;168:685–90. 23. Knott-Craig CJ, Overholt ED, Ward KE, Razook JD. Neonatal repair of Ebstein’s anomaly: indications, surgical technique,
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and medium-term follow-up. Ann Thorac Surg 2000;69: 1505–10. Reemtsen BL, Fagan BT, Wells WJ, Starnes VA. Current surgical therapy for Ebstein anomaly in neonates. J Thorac Cardiovasc Surg 2006;132:1285–90. Reemtsen BL, Polimenakos AC, Fagan BT, Wells WJ, Starnes VA. Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly. J Thorac Cardiovasc Surg 2007;134:1406–10; discussion 1410–2. Raju V, Dearani JA, Burkhart HM, et al. Right ventricular unloading for heart failure related to Ebstein malformation. Ann Thorac Surg 2014;98:167–73; discussion 173–64. Huang SC, Ishino K, Kasahara S, Yoshizumi K, Kotani Y, Sano S. The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa. J Thorac Cardiovasc Surg 2009;138:1160–6. Sano S, Ishino K, Kawada M, Fujisawa E, Kamada M, Ohtsuki S. Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum. Ann Thorac Surg 2000;70:1501–6. Ashburn DA, Blackstone EH, Wells WJ, et al. Determinants of mortality and type of repair in neonates with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 2004;127:1000–7; discussion 1007–8.
DISCUSSION DR T. K. SUSHEEL KUMAR (Memphis, TN): First of all, I want to congratulate you and your team on an excellent presentation, and thank you for sending us a copy of your manuscript and the presentation well ahead of time. As we all know, Ebstein’s anomaly is a rare congenital heart disease and management in the symptomatic neonate can be a medical and a surgical challenge. We also do know that Ebstein’s anomaly is just not an isolated disease of the tricuspid valve. It is also a disease of the right ventricular myocardium, often with significant atrialization of the right ventricle and small functional right ventricle. The condition carries significant mortality in the symptomatic neonates and is uniformly fatal without timely surgical intervention when conservative measures fail. The surgical approach to the symptomatic neonate has varied widely between an aggressive biventricular repair as advocated by Dr Christopher Knott-Craig and a single ventricle approach as pioneered by Dr Vaughn Starnes. Now, the critics of the biventricular approach will point out that the right ventricle may not have sufficient capacity to handle the cardiac output, while critics of the single ventricle approach will argue that by causing functional tricuspid atresia, one has taken away the opportunity for the right ventricle to grow. A recent survey in the United States shows the wide variation in practice with regard to a symptomatic neonate. Against this background, your concept of a staged biventricular management adds a new approach to a very difficult problem. Your biventricular approach, as you mentioned in the manuscript, is based on the concept of management of pulmonary atresia with intact ventricular septum and has worked well for your patients. Now, before going to my questions, I have to say that by combining tricuspid valve replacement with relief of pulmonary outflow tract obstruction and the addition of atrial septal defect (ASD) and Blalock-Taussig shunt, you are reducing the burden on the right ventricle. In a way, you are giving the right ventricle an opportunity to declare itself. If the right ventricle grows and begins to take on more of the cardiac output, the patient can head toward a two ventricle repair. Maybe that could be achieved in the cardiac catheterization lab.
Now, as against that, if the right ventricle does not grow, the patient could be taken along the single ventricle pathway, including upsizing of shunt if needed, Glenn or Fontan. So I think those are the merits of your approach. Now, although the concept is very attractive, there are some inherent flaws which I would like to point out before going into the questions. The right ventricular myocardium in the pulmonary atresia intact ventricular septum, you will agree with me, is completely different from the myocardium in an Ebstein’s patient. So it would be wrong to compare the growth potentials of the myocardium in two different conditions. A dilated right ventricle with residual tricuspid regurgitation and possible pulmonary regurgitation can compromise early function. However, as your results show, some of these fears are unfounded. My questions are here. The first one is regarding the cone technique. The performance of a cone technique in a neonate can be technically challenging. As you know, the original technique was described by Dr da Silva and modified by Dr Joe Dearani by addition of a pericardial patch. Did you have to do that on any of your patients? Did you use the classic technique or did you use the modified technique? DR HUANG: Indeed, I started to do this kind of repair before I saw the paper by da Silva. Indeed, I got the experience from Professor Sano. And I did not have any experience of adding a pericardial patch, because in these 7 patients we could make a competent tricuspid valve by its own tissue. DR KUMAR: Thank you. My second question is about the association of pulmonary stenosis or atresia. As we know, sometimes up to half of the symptomatic patients with Ebstein’s anomaly can have functional or anatomic pulmonary atresia. Now, as our own group showed, results with the biventricular approach can be poor unless you have a valved conduit, and our own mortality with a transannular patch was 40% and this was reduced to 16% once we started using a valved conduit. And as you know, this may be related to the addition of extra volume on
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DR HUANG: In our country, we do not have a small homograft and we do not have the Contegra for reconstruction of the right ventricular outflow tract. We only have the Gore-Tex membrane with a handmade monocuspid method to make a pulmonary valve. So it becomes an issue for dealing with a patient requiring a pulmonary valve. It is quite a challenge in our hospital. DR KUMAR: I understand that. The third question relates to the conclusion that you made in the last slide. It says that 6 of your 7 patients achieved biventricular repair, but in 2 of your patients, as mentioned in the manuscript, the ASD is still large and the flow was right to left. I think it may be premature to declare successful biventricular repair on patients unless their ASD closed or the flow across the ASD is predominantly left to right. Do you have anything to say about that? DR HUANG: Yes, I agree that it is not completed yet, but we anticipate completing it. DR KUMAR: And the last question that I have relates to the demonstration of the video. I think a few of us had the opportunity to view the video 2 days back. I have to congratulate you once again on your excellent approach and the excellent quality of the video presentation. You combined your tetralogy of Fallot repair with an Ebstein’s, and that was very much appreciated. But as I pointed out that evening I would like to reiterate that you should also pay attention to whether you can preserve the pulmonary valve or not. If you cannot preserve the pulmonary valve, I think that you may not have had such a good result. Do you agree with me on that?
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DR HUANG: I understand in the neonatal cases, preserving a competent pulmonary valve and tricuspid valve is very important, in contrast to that in the late infancy or the adolescent. So if the pulmonary valve could not be competent, we need a very competent tricuspid valve after repair. This is the key for survival if we do not do a Starnes operation or single ventricle palliation. We do agree. DR KUMAR: Dr Huang, I want to congratulate you once again. I enjoyed reading the manuscript. I learned a lot from reading your manuscript. Thank you very much. DR BRIAN L. REEMTSEN (Los Angeles, CA): I was actually with Vaughn and wrote up most of his initial data on the Starnes technique, but my thoughts and my practice have evolved. One of the biggest reasons is that when these babies are born, even when they have wall-to-wall hearts, a lot of times if they do not have pulmonary stenosis, they can be medically treated and they can leave the hospital. I am trying to find a reason to operate on these kids, and they are doing well and they are getting out of the intensive care unit. I just want to know what were these severe symptoms these kids were having who just had severe tricuspid valve regurgitation and no pulmonary stenosis, because in our hospital, most of those patients, the grand majority, leave the hospital. DR HUANG: The same. In our hospital if the patient does not have a ventricular septal defect and severe tricuspid regurgitation or pulmonary valve stenosis or atresia, most of them were discharged without operation. The heart becomes smaller and they are discharged from the hospital without mortality. These particular patients I operated were because of severe tricuspid regurgitation and the functional pulmonary atresia, or with the Great Ormond Street index above 1. It predicts 100% mortality, so we go on to surgery. DR REEMTSEN: What I am saying is if they are on drips, they cannot get off the ventilator, what is making you operate? DR HUANG: No. All the patients could not separate from the ventilator, so we do the surgery. If the patient was separated from ventilator, the patient could be discharged.
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the right ventricle or creation of a circular shunt. As we also realize the only fatality that you pointed out in your group of patients had a transannular patch and possibly resulted in early volume overload. In your manuscript you mention among the limitations that there are no homografts available in your country. That is mentioned in the manuscript. Did you consider any alternatives like a valved CorMatrix or a valved polytetrafluoroethylene graft?
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